Olfactory Neuroblastoma Causing Ectopic ACTH Syndrome

Abstract

Abstract.A 39-year-old woman who presented with typical Cushingoid appearance (moon facies, central obesity, purpura) was admitted to our hospital because of pulmonary infection. She was found to have hypertension, severe hypokalemia, and metabolic alkalosis. Endocrine data revealed elevated plasma levels of ACTH and cortisol with lack of circadian rhythm, non-suppressibility to high-dose dexamethasone, and hyperresponsiveness to CRH stimulation. Although no pituitary mass was detected by MRI of the brain, inferior petrosal sinus sampling showed a step-up of central to peripheral ACTH levels; these data are consistent with the diagnosis of Cushing's disease. She was successfully treated with metyrapone to control hypercortisolemia. Ten months later, a mass was detected in the ethmoid sinus, which was surgically removed. After resection of the ethmoid sinus tumor, her Cushingoid features and hypercortisolemia disappeared, but recurred after enlargement of a second mass in the maxillary sinus. After resection of the maxillary sinus tumor, her hypercortisolemia subsided. Histologically, the tumor tissues from both the ethmoid and maxillary sinus were identical and consistent with the diagnosis of olfactory neuroblastoma. Immunohistochemically, the immunoreactivities of ACTH and POMC were positive in the cytoplasm of tumor cells, and immunoreactive ACTH was demonstrated in both tumor tissues. Thus, this is the second rare case with ectopic ACTH syndrome caused by olfactory neuroblastoma thus far reported.

Clinical features and management of ectopic ACTH syndrome at a single institute in Japan

“…Although there are several possible causes of false-negative findings mainly due to anatomical abnormalities of venous drainage, false-positive cases have been rarely reported [26,27]. In our series, two cases showed positive result of IPS sampling (Case 4) and normal response to CRH stimulation (Case 8), which may be partly explained by tumor venous drainage closest to the cavernous sinus [5] and the possible trough period of cyclic hormonosingle institution in Japan.…”

“…Diagnostic imaging test by SRS not available in Japan yet was approved by the Ethics Committee of TMDU Hospital; written informed consent was obtained from each patient. Some cases have been reported elsewhere [5][6][7][8][9][10].…”

“…Among 10 cases who underwent IPS/CS sampling, 8 (80%) showed no ACTH gradient before and after CRH stimulation except for two; Case 4 (olfactory neuroblastoma) with C/P ratio (2.8) under the basal state [5] and Case 8 (pulmonary carcinoid tumor) with C/P ratio (3.5) after CRH stimulation [9].…”

Intraoperative Hypertensive Crisis Due to a Catecholamine-Secreting Esthesioneuroblastoma

“…Other studies have reported secretion of adrenocorticotropic hormone (ACTH) from esthesioneuroblastomas (17)(18)(19)(20)(21)(22)(23)(24) . Syndrome of inappropriate antidiuretic hormone (SIADH) has also been reported in patients with esthesioneuroblastoma (9,(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36) .…”

Esthesioneuroblastoma (Olfactory Neuroblastoma) with Ectopic ACTH Syndrome: A Multidisciplinary Case Presentation from the Joan Karnell Cancer Center of Pennsylvania Hospital

“…Eight cases have previously been described [22][23][24][25][26][27][28]. Ectopic ACTH production, stimulating cortisol secretion and producing Cushing's syndrome, is most commonly a result of small cell lung carcinomas, bronchial carcinoids, and islet cell tumors [29,30].…”